Incidence of and Risk Factors for Sudden Cardiac Death in Children with Dilated

Cardiomyopathy: A Report from the Pediatric Cardiomyopathy Registry

Elfriede Pahl, MD, FACC; Lynn A. Sleeper, ScD; Charles E. Canter, MD, FACC; Daphne T. Hsu, MD, FACC; Minmin Lu, MS;

Steven A. Webber, MBChB, FACC; Steven D. Colan, MD, FACC; Paul F. Kantor, MBBCh, FRCPC; Melanie D. Everitt, MD;

Jeffrey A. Towbin, MD, FACC; John L. Jefferies, MD, FACC; Beth D. Kaufman, MD, FACC; James D. Wilkinson, MD, MPH;

Steven E. Lipshultz, MD

for the Pediatric Cardiomyopathy Registry Investigators

Funding

Supported by grants from the National Heart, Lung, and Blood Institute HL53392 and the Children’s Cardiomyopathy Foundation.

The contents of this publication are solely the responsibility of the authors and do not necessarily represent the official views of the NHLBI.

Clinical Trials Registration # NCT00005391

Objective

To establish the incidence of, and risk factors for, SCD in pediatric DCM.

J Am Coll Cardiol 2012;59:607-15

Background

The incidence of SCD in children with DCM is unknown.

The ability to predict patients at high risk for SCD will help define who may benefit most from ICDs.

J Am Coll Cardiol 2012;59:607-15

Methods

The cohort was 1803 children in the PCMR diagnosed with DCM from 1990-2009.

Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using CART methodology.

J Am Coll Cardiol 2012;59:607-15

Results Five-year incidence rates were 29% for heart transplant, 12.1%

non-SCD, 4.0% death from unknown cause, and 2.4% for SCD. Of 280 deaths, 35 were SCD and cause was unknown for 56. The 5-year rate for SCD incorporating a subset of the unknown

deaths is 3%. Patients receiving anti-arrhythmic medication were at higher risk

of SCD (HR 3.0, 95% CI 1.1-8.3, p = 0.025). A risk stratification model based on most recent echocardiographic values had 86% sensitivity and 57% specificity.

Thirty of 35 SCDs occurred in patients who met all of these criteria: LV end-systolic dimension z score > 2.6, age at diagnosis <14.3 years, and ratio of LVPWT:EDD <0.14.

Sex, ethnicity, cause of DCM, and family history were not associated with SCD.

J Am Coll Cardiol 2012;59:607-15

Competing risks analysis for sudden cardiac death, non-sudden cardiac death, unknown cause of death and cardiac transplantation among 1803 children with dilated cardiomyopathy (DCM) listed in the Pediatric Cardiomyopathy Registry

J Am Coll Cardiol 2012;59:607-15

Classification and Regression Tree for SCD: Predictors from Time of Cardiomyopathy Diagnosis (Total N = 1747, 35 SCDs). Red boxes denote high-risk patient groups. Bold black boxes denote lower-risk patient subgroups. Sensitivity = 57%, specificity = 78%. False positive rate = 95%, false negative rate = 1.1%.

J Am Coll Cardiol 2012;59:607-15

Classification and Regression Tree for SCD: Predictors from time of last follow-up (Total N=1747, 35 SCDs).

Red box denotes the high-risk patient group. Bold black boxes denote lower-risk patient subgroups. Sensitivity = 86%, Specificity = 57%. False positive rate = 96%, false negative rate = 0.6%.

J Am Coll Cardiol 2012;59:607-15

Conclusions

The 5-year incidence of SCD in children with DCM is 3%.

A risk stratification rule (86% sensitivity) included diagnosis age <14.3 years, LV dilation, and LV posterior wall thinning.

Patients who consistently meet these criteria should be considered for ICD placement.

J Am Coll Cardiol 2012;59:607-15